title: Essential thrombocythemia: a rare disease in childhood.
abstract: Essential thrombocythemia is an acquired myeloproliferative disorder characterized by the proliferation of megakaryocytes in bone marrow, leading to a persistent increase in the number of circulating platelets and thus increasing the risk for thrombotic and hemorrhagic events. The disease features leukocytosis, splenomegaly, vascular occlusive events, hemorrhages and vasomotor disorders. The intricate mechanisms underlying the molecular pathogenesis of this disorder are not completely understood and are still a matter of discussion. Essential thrombocythemia is an extremely rare disorder during childhood. We report on a case of essential thrombocythemia in a child and discuss the diagnostic approach and treatment strategy.
authors.affiliation: Universidade de São Paulo - USP, São Paulo, SP, Brazil.
authors.forename: Julia Maimone
authors.initials: JM
authors.lastname: Beatrice
authors.forename: Marlene Pereira
authors.initials: MP
authors.lastname: Garanito
keywords: Case reports; Child; Female; Humans; Hydroxyurea/therapeutic use; Myelodysplastic-myeloproliferative diseases; Platelet aggregation inhibitors/therapeutic use; Thrombocythemia, essential/drug therapy; Thrombocytosis; Thrombosis